In 1930, Courville defined the term ganglioglioma as tumors consisting of astrocytic neuronal components with rare mitotic figures (see Figure 8).140 Approximately 50% show focal areas of calcification. Generally, they are benign growths that occur over a long clinical course and present with no specific radiologic characteristics.144
Figure 8. Ganglioglioma. 200x Photomicrograph of an admixture of mildly atypical neoplastic glial cells and neurons.
Most are located supratentorially, often in the temporal lobes.143 In one study of 137 patients, 65% were located in the frontal, temporal, or frontotemporal regions, versus only 8% in the midline or deep structures.141(See Figure 2.)
In a study by Otsubo and colleagues, 59% of patients presented with seizures. Of these, 64% had complex partial seizures, 32% had secondarily generalized seizures, and 4% presented with generalized seizures.
Optimal treatment includes gross total resection. Adjuvant chemotherapy or radiation therapy is generally not indicated.38
Postoperative 5-year survival of hemispheric gangliogliomas has been reported as 93%, although seizure relief statistics were lower than expected.145 Morris and colleagues reported freedom from seizures in 79% (30 of 38) of patients at 6 months, and 72% at 1 year postoperatively.137 Favorable prognostic factors included younger patient age, benign histopathology, stable clinical status at admission, decreased duration of seizure disorder, and lack of generalized features of seizures.137,141
Another group reported that among patients who presented with neurologic deficits or symptoms of increased intracranial pressure, those who were given adjuvant radiation had a postoperative 5-year survival of 53% versus 11% for those who did not receive radiation.141
Rarely, these tumors (as well as dysembryoplastic neuroepithelial tumors [DNETs]) may be associated with a rare postoperative disorder, with schizophreniform features of paranoia, depression, and psychosis. This psychosis does not seem to accompany other tumor types resected for intractable epilepsy.151,152
Reviewed and revised March 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.
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